Başaran Bora, Orhan Kadir Serkan, Polat Beldan, Mete Ozgür, Başerer Nermin
İstanbul Üniversitesi İstanbul Tıp Fakültesi Kulak Burun Boğaz Hastalıkları Anabilim Dalı, İstanbul, Türkiye.
Kulak Burun Bogaz Ihtis Derg. 2011 Jul-Aug;21(4):220-4. doi: 10.5606/kbbihtisas.2011.030.
Malignant peripheral nerve sheath tumors arising from the parotid gland are very rare. They can develop as sporadic cases, or on the basis neurofibromatosis type 1. Tumors originating from the parotid gland are generally easy to palpate and have a solid characteristic. Even if the tumor is malignant in character, the incidence of facial paralysis at the time of diagnosis is around 15% in various studies. However, a malignant tumor originating from the nerve itself may not be noticed during the physical examination for a long period time although it cases facial paralysis and may be mistaken with other non-neoplastic diseases involved in the etiology of facial paralysis leading to a delay in the diagnosis and treatment. Especially patients with type 1 neurofibromatosis have a great tendency to develop malignant peripheral nerve sheath tumors. In this article a case of malignant peripheral nerve sheath tumor developed on the basis of neurofibromatosis type 1 was reported.
起源于腮腺的恶性外周神经鞘瘤非常罕见。它们可作为散发病例出现,或在1型神经纤维瘤病的基础上发生。起源于腮腺的肿瘤通常易于触及,具有实性特征。即使肿瘤为恶性,在各种研究中,诊断时面瘫的发生率约为15%。然而,起源于神经本身的恶性肿瘤,尽管导致面瘫,但在体格检查中可能长期未被发现,可能被误诊为其他与面瘫病因相关的非肿瘤性疾病,从而导致诊断和治疗延迟。特别是1型神经纤维瘤病患者极易发生恶性外周神经鞘瘤。本文报道了1例在1型神经纤维瘤病基础上发生的恶性外周神经鞘瘤病例。